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Imaging evaluation of hereditary renal tumors: a pictorial review

Authors
  • Tanaka, Takashi1
  • Kawashima, Akira2
  • Marukawa, Yohei1
  • Kitayama, Takahiro1
  • Masaoka, Yoshihisa1
  • Kojima, Katsuhide1
  • Iguchi, Toshihiro1
  • Hiraki, Takao1
  • Kanazawa, Susumu1
  • 1 Okayama University Hospital, 2-5-1 Shikata-cho, Okayama, 700-8558, Japan , Okayama (Japan)
  • 2 Mayo Clinic, 13400 E Shea Blvd, Scottsdale, AZ, 85259, USA , Scottsdale (United States)
Type
Published Article
Journal
Japanese Journal of Radiology
Publisher
Springer Singapore
Publication Date
Mar 23, 2021
Volume
39
Issue
7
Pages
619–632
Identifiers
DOI: 10.1007/s11604-021-01109-5
Source
Springer Nature
Keywords
Disciplines
  • Invited Review
License
Yellow

Abstract

More than 10 hereditary renal tumor syndromes (HRTSs) and related germline mutations have been reported with HRTS-associated renal and extrarenal manifestations with benign and malignant tumors. Radiologists play an important role in detecting solitary or multiple renal masses with or without extrarenal findings on imaging and may raise the possibility of an inherited predisposition to renal cell carcinoma, providing direction for further screening, intervention and surveillance of the patients and their close family members before the development of potentially lethal renal and extrarenal tumors. Renal cell carcinomas (RCCs) associated with von Hippel–Lindau disease are typically slow growing while RCCs associated with HRTSs, such as hereditary leiomyomatosis and renal cell carcinoma syndrome, are highly aggressive. Therefore, radiologists need to be familiar with clinical and imaging findings of renal and extrarenal manifestations of HRTSs. This article reviews clinical and imaging findings for the evaluation of patients with well-established HRTSs from a radiologist’s perspective to facilitate the clinical decision-making process for patient management.

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